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ID 10580
Eprint ID
10580
FullText URL
Thumnail 93_73_76.pdf 1.64 MB
Title Alternative
Expression of Evc and Lbn Responsible for Ellis-van Creveld Syndrome in Tibial Growth Plate
Author
Abstract
Ellis-van Creveld (Evc) syndrome is an autosomal recessive chondrodysplasia characterized by disproportionate dwarfism, postaxial polydactyly, natal teeth and dysplatic nails. Ellis-van Creveld (EVC) gene, which is mutated in patients with Evc syndrome, has recently been identified by positional cloning. LIMBIN(LBN), responsible for bovine chondrodysplastic dwarfism, is also mutated in patients with Evc syndrome. However, the physiological role of EVC and LBN have not been elucidated. To assess the role of Evc and Lbn in the rat tibia were most abundant at embryonic day 1, 28 and 56. The analysis by in situ hybridization revealed that the Evc was expressed mainly in prehypertrophic and hypertrophic chondrocytes in the rat tibial growth plate at postnatal stage. Lbn was detected strongly in proliferative chodnrocyte and moderately in prehypertrophic chondrocytes. These profiles of Evc and Lbn expression reflected the abnormalities in chondrocytes of patients with Evc syndrome. Our results suggest that Evc and Lbn may affect chondrocyte differentiation and proliferation in endochondral ossification, respectively.
Keywords
chondrocyte
EVC
LBN
Ellis-van Creveld syndrome
endochondral ossification
Published Date
2004-02
Publication Title
岡山大学農学部学術報告
Publication Title Alternative
Scientific reports of the Faculty of Agriculture, Okayama University
Volume
volume93
Issue
issue1
Publisher
岡山大学農学部
Publisher Alternative
Faculty of Agriculture,Okayama University
Start Page
73
End Page
76
ISSN
0474-0254
NCID
AN00033029
Content Type
Departmental Bulletin Paper
language
日本語
File Version
publisher
Refereed
False
Eprints Journal Name
srfa